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This gene encodes a member of the sedolisin family of serine proteases. The protease functions in the lysosome to cleave N-terminal tripeptides from substrates, and has weaker endopeptidase activity. It is synthesized as a catalytically-inactive enzyme which is activated and auto-proteolyzed upon acidification. Mutations in this gene result in late-infantile neuronal ceroid lipofuscinosis, which is associated with the failure to degrade specific neuropeptides and a subunit of ATP synthase in the lysosome.
cell growth-inhibiting gene 1 protein; ceroid-lipofuscinosis, neuronal 2; CLN 2; CLN2; GIG 1; GIG1; growth-inhibiting protein 1; LPIC; lysosomal pepstatin insensitive protease; lysosomal pepstatin-insensitive protease; MGC21297; SCAR7; TPP 1; TPP I; Tpp1; TPP-1; TPPI; TPP-I; Tripeptidyl aminopeptidase; tripeptidyl peptidase 1; tripeptidyl peptidase I; tripeptidyl peptidase-I; tripeptidyl-peptidase 1; Tripeptidyl-peptidase I; UNQ267/PRO304
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